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The protein encoded by HMGCL belongs to the HMG-CoA lyase family. Zusätzlich bieten wir Ihnen HMGCL Antikörper (109) und HMGCL Proteine (13) und viele weitere Produktgruppen zu diesem Protein an.
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Data suggest that HMGCS1 (HMG-CoA synthase (zeige HMGCS1 ELISA Kits) 1) signals through ketogenesis/acetoacetate to promote cell proliferation and BRAF (zeige BRAF ELISA Kits)(V600E)-dependent MEK1 (zeige MAP2K1 ELISA Kits) activation in BRAF (zeige BRAF ELISA Kits)(V600E)-positive melanoma and colon cancer cells; HMGCS1 (zeige HMGCS1 ELISA Kits) co-localizes with HMGCL (HMG-CoA lyase) and BRAF (zeige BRAF ELISA Kits)(V600E) in cytosol of melanoma and colon cancer cells. (BRAF (zeige BRAF ELISA Kits) = proto-oncogene (zeige RAB1A ELISA Kits) protein B (zeige LEPREL2 ELISA Kits)-raf (zeige SNRPE ELISA Kits))
The genetic analysis revealed a novel homozygote deletion in exon 3 and 4 in HMGCL gene. HMG-CoA lyase deficiency should be thought in the patients with hypoketotic hypoglycemia, hyperammonemia, elevated liver function tests, noncompaction left ventricle and characteristic white matter changes and in the differential diagnosis of macrocephaly.
this is the first study describing HMGCL deficiency caused by uniparental disomy.
This efficient UPLC-MS/MS assay permits rapid and high sensitive determination of HMGCR (zeige HMGCR ELISA Kits) enzyme activity, tracing potential alterations in cholesterol biosynthesis.
analysis of HMGCLL1 as an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase and comparison with MHGCL
An alternative transcript of HMGCS2 (zeige HMGCS2 ELISA Kits) carrying a deletion of exon 4, and two alternative transcripts of HMGCL with deletions of exons 5 and 6, and exons 5, 6 and 7, respectively, were detected.
levels of enzyme activity do not strongly correlate with formation of inter-subunit adducts by HMGCL mutants. C170S, C266S, and C323S proteins do not form inter-subunit disulfide adducts but such an adduct is restored in the C170S/C174S double mutant.
Crystal structures of ternary complexes of WT HMGCL with the competitive inhibitor 3-hydroxyglutaryl-CoA and of the catalytically deficient HMGCL R41M mutant with substrate HMG-CoA have been determined to 2.4 and 2.2 A.
We report on a new case of 3-hydroxy-3-methylglutaric aciduria particular by its late onset in a 3-year-old patient. Molecular investigation identified two new sequence modifications in the HMGCL gene: c.494G>A (p.Arg165Gln) and c.820G>A (p.Gly274Arg).
A role is suggested for arginine-41 in deprotonation or enolization of acetyldithio-CoA, implicating this residue in the HMG (zeige SSRP1 ELISA Kits)-CoA cleavage reaction chemistry that leads to acetyl-CoA (zeige LPCAT2 ELISA Kits) product formation.
The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase
, 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
, 3-hydroxy-3-methylglutarate-CoA lyase
, HMG-CoA lyase
, hydroxymethylglutaryl-CoA lyase, mitochondrial
, 3-hydroxy-3-methylglutaryl-CoA lyase
, mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase
, 3-hydroxy-3-methylglutaryl-Coenzyme A lyase